全反式维甲酸治疗急性早幼粒细胞白血病并发Sweet's综合征(附一例报告并文献复习)

Development of Sweet syndrome in an acute promyelocyte leukemia patient during treatment with alltrans retinoic acid——case report and literature review

目的 讨论并鉴别全反式维甲酸（ATRA）引起的少见的不良反应，提高急性早幼粒细胞白血病（APL）早期治疗的效果。方法 报告国内首例ATRA治疗APL并发Sweet’s综合征的病例，并进行相关文献复习。结果 文献报道共有13例APL患者发生与ATRA治疗相关的Sweet’s综合征。包括本例在内的14例患者，中位年龄49．5（9～84）岁，女10例，男4例。10例患者单独累及皮肤，4例除皮肤外还累及肌肉、肺部、肾脏。皮肤累及以上肢为多（11例），其他部位包括下肢、面部、颈部、背部、腹部、会阴部等。患者出现Sweet’s综合征时，ATRA治疗中位时间为18（6～34）d，外周血中位白细胞计数为7．05（0．80—23．00）×10^9／L。有4例患者未中断ATRA治疗，共有13例患者给予糖皮质激素治疗，12例有效。1例患者自发缓解。在全部14例患者中，有2例出现维甲酸综合征。结论 Sweet’s综合征为ATRA治疗APL患者过程中少见的并发症，与皮肤组织的炎症和感染不易鉴别，其发生的机制与维甲酸综合征之间的关系尚不清楚。糖皮质激素治疗有效，再次应用ATRA后未见复发。

Objective To identify the side effect of all-trans retinoic acid （ATRA）, and improve early therapeutic response in patients with acute promyelocytic leukemia （APL）. Method The first case of Sweet＇ s syndrome（SS） developed in a APL patient treated with ATRA was reported in mainland of China, and reviewed correlative literature. Results Only 14 cases of SS associated with ATRA therapy in APL have been reported in the literature, including the present case. The median age was 49.5 years （9 -84） and 10 were women and 4 men. Of them, SS was restricted to the skin in 10 case, the other 4 muscle, fascia, kidney, and lung were involved. SS appeared after a median of 18 days of ATRA therapy （6 -34 days）. The median WBC count was 7.05 （0.80 -23.00）× 10^9/L. Four patients continued with the ATRA therapy without interruption, 13 patients treated with steroids and 12 responded. One patient improved without any treatment. Two cases of SS developed retinoic acid syndromes after ATRA therapy. Conclusion Sweet＇ s syndrome is a rare adverse effect of ATRA, and has similar features with inflammatory or infective dermatosis. The corticosteroids treatment could improve the systemic and cutaneous symptoms. When ATRA therapy was restarted after SS subsided, no recurrence of rashes was observed.